[Successful treatment of adult Still's disease with tofacitinib in a HIV-2 positive female patient]. / Erfolgreiche Behandlung eines adulten Morbus Still mit Tofacitinib bei einer HIV-2-positiven Patientin.

A 46-year-old female patient with a known HIV-2-infection suffered from adult onset Still's disease, which was initially complicated by a macrophage activation syndrome (MAS). The required glucocorticoid treatment induced a psychosis and the patient developed an aversion to glucocorticoids. After failure of treatment with anakinra, an alternative option with the JAK-inhibitor tofacitinib was introduced because of the short half-life and to reduce glucocorticoid exposure. A switch to tofacitinib was only successful after an overlapping treatment with anakinra and tofacitinib for 3 weeks. The patient is currently being treated with monotherapy with tofacitinib as well as NSAID on demand, is in stable remission and can continue working as normal.

[Auricular chondritis as first symptom of ANCA-associated vasculitis]. / Aurikuläre Chondritis als Erstsymptom einer ANCA-assoziierten Vaskulitis.

Auricular chondritis frequently occurs in relapsing polychondritis. In addition to the primary form of the disease up to 30% of cases of chondritis can be secondary, e.g. due to autoimmune diseases. We describe the case of a 62-year-old male patient with auricular chondritis as the first symptom of granulomatosis with polyangiitis. Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis should always be considered in the differential diagnostics of relapsing polychondritis and antibody testing should be performed accordingly.

Vertebral arteries: a target for FDG-PET imaging in giant cell arteritis? Clinical, ultrasonographic and PET study in 46 patients.

AIM: To demonstrate the capacity of FDG-PET to show active giant cell arteritis (GCA) of the extracerebral vertebral artery (VA) and to compare it with clinical, ultrasonographic (US) and biopsy findings. PATIENTS, METHODS: Observational study of 46 consecutive patients with the diagnosis of active GCA and abnormal high FDG uptake in the aorta and other large arteries suggestive for GCA. RESULTS: 15 of the 46 GCA patients had abnormal high FDG uptake within the extending from the V0 to V3 segment in 13 and confined to single segments in 2 patients. In 2 patients high FDG uptake in one VA was the onIy PET abnormality. In 13 patients high FDG uptake was also found in other large arteries (carotid n = 10, subclavian/axillary artery n = 12, thoracic aorta n = 12). Abnormal PET was detectable in 5 patients despite glucocorticoid (GC) treatment. Nuchal and occipital pain and ischemic stroke or TIA in the posterior circulation (n = 3) were found in 10 patients with high VA FDG uptake. US detected halos of the V0-2 segments in 8/46 patients (5/15 VA PET positive and 3/31 VA PET negative patients). Biopsies were available in 10/15 VA PET positive patients with evidence of active GCA in 7 cases. CONCLUSION: In patients with severe GCA and a high TVS the extracranial VA are a good target for PET imaging in active GCA with abnormal findings in 33% of patients with a positive PET. VA abnormalities can be an early and isolated finding in active GCA. PET is superior to US for the detection of active VA-GCA. A strong correlation between VA abnormalities and associated clinical abnormalities existed in 2/3 of patients. PET abnormalities of the VA could be detected in some cases after GC treatment has been started even at high doses.

[Chronic anaemia in a patient with hereditary haemorrhagic telangiectasia and juvenile gastric polyposis]. / Chronische Anämie bei hereditärer hämorrhagischer Teleangiektasie und juveniler Polyposis des Magens.

HISTORY: A 52-year-old woman with chronic anaemia presented with recurrent epistaxis, telangiectasias and haemangiomas, suggesting the presence of hereditary haemorrhagic telangiectasia (Osler-Rendu-Weber disease). Moreover, previous investigations had also shown multiple polyps of the stomach. INVESTIGATIONS: A severe microcytic iron deficiency anaemia in combination with hepatic haemangiomas and duodenal angio-dysplasia were detected. Gastrointestinal endoscopy revealed multiple juvenile polyps in the gastric fundus and body, indicating the diagnosis of hereditary haemorrhagic telangiectasia in combination with juvenile polyposis. THERAPY AND COURSE: A gastrectomy was performed because of recurrent gastrointestinal bleeding and the malignant potential of juvenile gastric polyposis. Histopathology confirmed the diagnosis and did not reveal any malignancy. CONCLUSION: The association of hereditary haemorrhagic telangiectasia and juvenile polyposis, as seen in this patient, has been reported repeatedly. A diagnosis of one of the described entities should initiate the screening for evidence of the other one to prevent (life-threatening) complications.

Dietl's crisis: a syndrome of episodic abdominal pain of urologic origin that may present to a gastroenterologist.

A 53-yr-old woman presented with a 2-yr history of recurrent episodes of severe abdominal pain and nausea. Multiple investigations by a general surgeon, a urologist, and a gastroenterologist failed to identify the cause. She was referred to our Biliary Service for ERCP and sphincter of Oddi manometry. However, a detailed history was inconsistent with biliary pain, and the patient, having discussed the risks and benefits, elected not to proceed with ERCP. The patient was asked to come to the hospital during an acute attack of her pain for assessment. When this was done, transabdominal ultrasound revealed right hydronephrosis; intravenous urography showed obstruction at the level of the ureteropelvic junction, consistent with the presence of an aberrant artery. The syndrome of episodic abdominal pain and hydronephrosis caused by extrinsic pressure from such an artery is known as Dietl's crisis. In our patient, the diagnosis was confirmed at surgery, when the ureteric obstruction was dealt with by pyeloplasty. She made an uneventful recovery and remains asymptomatic 12 months later. The keys to diagnosing Dietl's crisis are awareness of the entity, taking a detailed pain history, and timely cross-sectional abdominal imaging during an attack.

Enormous hemangiosarcoma of the heart.

This report describes a 26-year-old patient with hemangiosarcoma of the heart and summarizes the clinicopathological features in previous reports of patients with cardiac angiosarcoma. The patient was admitted to our hospital because of a syncope and one episode of nocturnal dyspnea and hemoptysis. In his history he complained of progressive weakness and loss of weight over the past 2 months. Echocardiography and computed tomography of the chest showed inhomogeneous masses in the pericardial cavity completely surrounding the heart and involving the ascending aorta and the superior vena cava. Histological examination of the tissue obtained from the mass by fine needle technique revealed a poorly differentiated malignant tumor of mesenchymal origin. Exploratory thoracotomy followed by tumor biopsies showed an inoperable cardiac hemangiosarcoma of enormous size with multiple metastases in both lungs. Palliative tumor resection was not performed. During the postoperative course the patient still required controlled ventilation. After 3 days of cytostatic chemotherapy no regression of tumor mass was seen by chest radiography. Cardiorespiratory insufficiency was progressive, and the patient died within 3 weeks after admission.

Would revision arthroplasty be facilitated by extracorporeal shock wave lithotripsy? An evaluation including whole bone strength in dogs.

Extracorporeal shock-wave lithotripsy has been proposed as a modality to facilitate the removal of bone cement during revision arthroplasty; however, concomitant cortical microfractures have been reported. The current study examines the effect on whole bone strength of extracorporeal shock-wave lithotripsy directed at the cement-bone complex. Canine femora were subjected to manual cement extraction or lithotripsy followed by manual cement extraction. Contralateral femora served as controls. Torsional fractures were created, and maximum torque, maximum angular displacement, and energy capacity to failure were determined. Although cement extraction alone reduced mean torque by 6.6% and failed to reduce mean torque angle or mean energy capacity, the combination of lithotripsy and cement extraction reduced mean torque by 7.3%, mean torque angle by 14.3%, and mean energy capacity by 18.3%. No statistical significance was demonstrated between the two groups in torque, angle, or energy capacity. At magnitudes and numbers of shock waves previously shown to significantly reduce cement-bone interface mechanical strength, lithotripsy exposure had a minimal and insignificant effect on whole bone strength.

The effect of extracorporeal shock wave lithotripsy on the prosthesis interface in cementless arthroplasty. Evaluation in a rabbit model.

The effect of extracorporeal shock wave lithotripsy on interfacial strength between prosthesis and bone in cementless arthroplasty was examined using a rabbit model. Paired femora, each implanted with fiber mesh porous coated titanium implants, were harvested from rabbits 15 weeks after implantation. In group I, one femur from each pair was exposed to lithotripsy treatment consisting of 2,000 shocks at 20 kV. In group II, one femur from each pair was exposed to 2,000 shocks at 26 kV. Contralateral femora from each pair served as controls in both groups. Mechanical pushout tests were conducted on the implants using a 1321 Instron testing machine at a constant rate of 1 mm/minute. Shock waves generated at 20 kV were found to have no significant decrease on either the prosthesis/bone interfacial strength or energy to failure of cementless implants. Shock waves generated at 26 kV produced a mean 17.45% decrease in the prosthesis/bone interfacial strength, which approached statistical significance (P = .062), and a 7.84% mean decrease in the energy to failure (P = .268). However, in four of the seven group II specimens, cortical fractures occurred. These findings suggest that lithotripsy will not aid in the removal of uncemented porous coated devices and lithotripsy inadvertently focused at an uncemented device will not disrupt significantly the prosthesis-bone interface.

Endoscopic laser lithotripsy of large bile duct stones.

Experimental work has established that the Candela (Candela Laser Corporation, Wayland, MA) flashlamp excited dye laser (wavelength, 504 nm) is a highly effective method for fragmenting biliary stones and has minimal potential for injuring the bile duct wall. This technique was evaluated in 25 complex patients whose stones, usually because of large size, did not respond to standard nonoperative treatment. The laser imaging was applied through a quartz fiber and aimed either under direct vision with choledochoscopes passed percutaneously or through a special "mother" duodenoscope or under fluoroscopic guidance at standard duodenoscopy. Laser treatment resulted in some fragmentation of stones in 23 cases. Subsequently, it proved that it was possible to clear the bile duct of stones in 20 patients, 12 of them receiving successful treatment during the same endoscopic procedure. There were no significant complications. This endoscopic technique seems to be a useful new alternative to surgery in patients with large and difficult bile duct stones.

Lessons learned in patients with large steinstrasse.

Experience with 19 patients (25 ureters) who suffered the complication of a large steinstrasse (a third or more of the ureteral length) has led to lessons regarding the management of this complication as well as to insights into patient presentation, metabolic consequences, and the efficiency of diagnostic and therapeutic modalities. Few symptoms were present in a large percentage of patients despite urinary obstruction, decreased renal function, infection and in several cases impending sepsis. Large stone burdens, bilateral treatment, inability to debulk stone burden before extracorporeal shock wave lithotripsy and unexpected fragment movement were definite predisposing factors in the development of this complication. Double pigtail stents and percutaneous nephrostomy alone did not always prevent or resolve the problem. Combined use of percutaneous nephrostomy and ureteroscopic ultrasonic or laser lithotripsy appears to be the most effective treatment modality in patients who fail brief observation. This approach can be combined under appropriate circumstances with a secondary extracorporeal shock wave lithotripsy or percutaneous nephrolithotripsy treatment in the staged management of complex upper urinary tract calculous disease.

Percutaneous transhepatic ultrasonic cholelithotripsy.

In a patient with acute cholecystitis whose body habitus precluded cholecystectomy, we used percutaneous transhepatic ultrasonic cholelithotripsy. We compare it with other modalities applicable in such cases. Although not commonly used, these alternatives may be the most appropriate therapy in certain unusual circumstances.

Role of linear tomography in evaluation of patients with nephrolithiasis.

One hundred twenty-four consecutive patients with suspected or known nephrolithiasis were referred for evaluation by linear tomography. Renal calculi were detected in 98 patients (79%). Linear tomography revealed more renal calculi than did preliminary KUB films in 46 patients (37%), although in only 10 cases (8%) was the tomogram positive if the KUB was negative. Exact quantification of the numbers and locations of renal stones is important in patients to be managed metabolically, and in those being evaluated in advance of or following percutaneous or extracorporeal lithotripsy.

Percutaneous lithotripsy in morbid obesity.

Percutaneous lithotripsy is an established, safe, effective method for the management of renal calculi. Obesity long has been associated with increased surgical morbidity and may eliminate a patient from shock wave treatment. We compared 44 obese patients to 226 nonobese patients undergoing percutaneous stone management. Stone number, location and total stone burden were comparable in the obese and nonobese groups. There was no significant difference between the groups in hospital time, operative time, fragment rate, access success rate or morbidity. Percutaneous procedures offer successful alternatives with low morbidity for patients with renal calculous disease.

Extracorporeal shock wave lithotripsy: the use of chemical treatments for improved stone comminution.

Extracorporeal shock wave lithotripsy (ESWL) can require more than two thousand acoustic shocks to achieve an adequate degree of renal calculus comminution. A decrease in the number of shocks necessary for effective treatment offers both technical and clinical benefits. The results presented here demonstrate that it is possible in particular cases to increase substantially the degree of comminution produced using a fixed number of acoustic impulses by exposing the stones to solutions of controlled pH and chemical composition during acoustic shock treatment. The largest increase in comminution was observed for magnesium ammonium phosphate hexahydrate/apatite stones exposed to citrate solutions. The smaller particle sizes are shown to result not only from stone dissolution but also from an increase in the ease of stone fracture during acoustic shocking. The degree of comminution of the largest fragment sizes was also found to be slightly increased for calcium oxalate stones by exposure to synthetic urine of elevated pH. These chemical methods of increased stone comminution appear to be directly applicable to particular cases and may have general clinical utility if suitable conditions affecting all stones can be found.

Intravenous digital subtraction angiography in the evaluation of potential renal donors.

Of 65 surgically removed donor kidneys intravenous digital subtraction angiography demonstrated accurately the number of renal arteries in 58 (89 per cent). All accessory vessels missed at digital subtraction angiography were small and their presence did not interfere with successful transplantation in those donated. Of 50 surgically removed donor kidneys examined with conventional aortography only before the routine use of intravenous digital subtraction angiography the number of renal arteries was demonstrated accurately in 46 (92 per cent). Intravenous digital subtraction angiography offers advantages over conventional aortography, including most importantly the routine performance on an outpatient basis, and decreased film cost and examination time. Although the accuracy of conventional aortography (92 per cent) in detecting the number of renal arteries is slightly greater than that for intravenous digital subtraction angiography (89 per cent), the advantages of the digital examination justify its use as the initial examination for the potential renal donor. Conventional aortography can be reserved for use in patients with equivocal or technically inadequate digital examinations.

Results and morbidity of percutaneous nephrolithotripsy.

The medical records of the first 210 patients treated with percutaneous nephrolithotripsy at Duke University Medical Center were reviewed. Eleven patients were treated for bilateral stones for a total of 221 renal units. The procedure failed in 6 patients (2.7%). Stone fragments were retained in 55 patients (24.9%), with unplanned fragments in 41 cases (18.6%). Open surgery was subsequently performed in 4 patients (1.8%). There were no nephrectomies or deaths in our patients. Percutaneous nephrolithotripsy is a highly successful method for removal of upper tract stones and has a complication rate no higher than that of open surgical procedures.

Lumbar hernia: diagnosis by CT.

Lumbar hernias occur in the region of the flank bounded by the 12th rib, the iliac crest, and the erector spinae and external oblique muscles. We present the CT findings of seven lumbar hernias: six traumatic (four secondary to postoperative flank incisions, one secondary to an iliac bone-graft donor site, one secondary to nonunion of an iliac fracture) and one spontaneous. Because CT portrays the anatomic relationships in this region so well, it may be the only radiographic procedure necessary to make the diagnosis of a lumbar hernia. Furthermore, it can be helpful in the assessment of symptomatic patients after flank incision, to differentiate postincisional muscular weakness and intercostal neuralgia from a lumbar hernia.